What is Hirschsprung's disease?

Also known as: Hirschsprung disease, megacolon

Hirschsprung's disease describes a congenital condition (happens before birth) where nerve cells in the wall of the large bowel (colon) that normally develop during intrauterine development are missing. These nerve cells are called ganglion cells and when missing from the intestinal wall the breakdown products of food are blocked from moving forward through the colon to the anus, and cannot be passed as stool.

It occurs in boys more often than girls and may be associated with some inherited conditions. The length of bowel that may be affected may be short or long and is frequently found in the end parts of the large bowel (upper rectum or sigmoid colon).


Reviewed by: Juan L Calisto, MD

This page was last updated on: 1/29/2019 3:21:13 PM

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